All seven of the present patients were females.440 W.A.D.GriffithsFIG'JRE 3. Whole biopsy. The extent of the maculc was marked on the surface with Indian ink(see arrows). Epidermal atrophy is seen between the arrows (H & E, x 25).FIGURE 4. [documents.tips]

Initially, thelesions were present only over the fingers and graduallythey increased in size as well as number to attain thepresent status. [docslide.com.br]

There is a single case report of DDD in an African- American woman presenting only as hypopigmented macules [ 2 ]. [omicsonline.org]

In addition, there were multiple palmar pits present with breaks in proximal palmer ridges. [idoj.in]

Case Presentation A 29-year-old male (Fitzpatrick Skin Type V) with unremarkable past medical history originally presented to the clinic with complaints of a nevus on the foot which was determined to be benign. [cureus.com]

• Asymptomatic

  The lesions were asymptomatic from thebeginning. There was no history of similar episodes in thepast or in the family, and no history of any skin lesions atbirth. [docslide.com.br]

  A 25-year-old male presented with asymptomatic dark-colored lesions on his hands and feet with light-colored skin lesions involving the trunk since three years. [ncbi.nlm.nih.gov]

  Although usually asymptomatic, pruritus of the affected areas may be the only symptom. [idoj.in]

  Al Hawsawi <strong>Reticulate</strong> <strong>acropigmentation</strong> <strong>of</strong> <strong>Kitamura</strong> (RAK) and Dowling-Degos Disease (DDD) are characterized by progressive symmetric and asymptomatic reticulated pigmented macules but [yumpu.com]

• Anemia

  […] syndrome *Partington syndrome *Atopic dirty neck *Epidermolysis bullosa with mottled pigmentation *Systemic sclerosis * Poikilodermatous *Inherited *Rothmund-Thomson syndrome *Dyskeratosis congenita *Xeroderma pigmentosum *Cockayne syndrome *Fanconi anemia [go.gale.com]

  Fanconi anemia Syn: Familial pancytopenia or familial panmyelophthisis This is a autosomal-recessive condition with five complementation groups (FA-A, FA-B, FA-C, FA-D, and FA-E) [43] which affect hematopoiesis. [ijdvl.com]

  Epidermolysis bullosa with mottled pigmentation Systemic sclerosis 3 Classification of Reticulate Dermatoses :Continue Poikilodermatous Inherited Rothmund-Thomson syndrome More Details Dyskeratosis congenita Xeroderma pigmentosum Cockayne syndrome Fanconi anemia [slideplayer.com]

  This theory is predicated on the increased frequency of a plethora of immunologic diseases in patients with vitiligo, including hypothyroidism (Hashimoto thyroiditis), Graves disease, pernicious anemia, diabetes mellitus, and alopecia areata. [what-when-how.com]

• Arthritis

  Dowling-Degos disease: Hidradinitis suppurativa and arthritis in mother and daughter. Clin Exp Dermatol 2006; 31(3): 454- 456. 6. Thami GP, Jaswal R, Kanwar AJ, Radotra BD, Singh IP. [edoj.org.eg]

  […] reticulate acropigmentation of Kitamura and Dowling-Degos disease. ( 25386285 ) Cabral A.R....Reis J.P. 2011 27 Exploring Rak tyrosine kinase function in breast cancer. ( 19597351 ) Yim EK...Lin SY 2009 28 Microchimerism of the shared epitope in rheumatoid arthritis [malacards.org]

• Hyperpigmentation

  is present at birth, or soon after,and shov/s spots, streaks and whorls of hyperpigmentation in all areas including the ocular and oralmucosae. [documents.tips]

  Figure 1: Reticulate hyperpigmentation on: (a) Dorsa of both hands (b) Dorsa of both feet (c) Neck (d) Axilla Click here to view Figure 2: Unusual features: (a) Reticulate hyperpigmentation in periorbital area (b) Reticulate hyperpigmentation on scrotum [doi.org]

  Reticulate hyperpigmentation on: (a) Dorsa of both hands (b) Dorsa of both feet (c) Neck (d) Axilla Unusual features: (a) Reticulate hyperpigmentation in periorbital area (b) Reticulate hyperpigmentation on scrotum (c) Hypopigmented macules on trunk [ncbi.nlm.nih.gov]

• Papule

  Initially, fewsmall brown coloured papules appeared over the chestwhich gradually increased in number and size to attain thepresent status. The lesions were asymptomatic from thebeginning. [docslide.com.br]

  Few darkly pigmented comedo-like papules and few atrophic scars were noticeable over upper back and neck [Figure 1] c. In addition, there were multiple palmar pits present with breaks in proximal palmer ridges. [idoj.in]

  Patients with the DDD-RAK spectrum may show hyperpigmented macules and papules, facial pits, breaks in dermatoglyphics and epidermoid cysts. [ncbi.nlm.nih.gov]

  Signs in the nails (DD, HHD), flat-topped warty papules on dorsa of hands and feet (DD) and palmar pits (DD, HHD) or palmar keratotic papules (DD) help to confirm the diagnosis. [accessmedicine.mhmedical.com]

  It is characterized by acquired reticulate hyperpigmentation over the flexures, comedone‐like follicular papules, and pitted… SHOWING 1-10 OF 15 REFERENCES [semanticscholar.org]

• Hypopigmented Macule

  Wu and Lin reported that hyperpigmented macule had lesser degree of acanthosis compared to the hypopigmented macule [ 3 ]. [omicsonline.org]

  In RAPK, there are usually no hypopigmented macules and histologically there is epidermal atrophy and increase in the number of basal melanocytes. [doi.org]

• Freckles

  It presents during adult life most frequently in the 4th decade as numerous, small, rounded pigmented macules that resemble freckles. [edoj.org.eg]

  On examination, she had irregular, atrophic, freckle-like macules on the neck, elbows, abdominal skin, and dorsa of the hands and feet (Fig. 5). It was noted that the freckle-like lesions were not as dark as in the mother. [escholarship.org]

  The lesions were irregularly angulated and freckle-like.They darkened gradually and extended proximally. [documents.tips]

  It first appears during the first or second decade of life, with reticulate or freckle-like hyperpigmented macules that measure 1 to 5mm. [actasdermo.org]

  Freckle-like macules are present on the face. Lesions appear in infancy or in early childhood and commonly stop spreading before adolescence. The pigmentation lasts for life. [thinkscience.in]

• Skin Disease

  Diseases, Genetic/genetics* Skin Diseases, Genetic/physiopathology Skin Diseases, Papulosquamous/genetics* Skin Diseases, Papulosquamous/physiopathology Young Adult Substances KRT5 protein, human Keratin-5 Membrane Proteins Amyloid Precursor Protein [ncbi.nlm.nih.gov]

  diseases (e.g., dyskeratosis, ichthyosis, epidermo lysis bullosa and pigmentation disorder), atopic dermatitis, urticaria, and other skin diseases. [med.nagoya-u.ac.jp]

  Homepage Rare diseases Search Search for a rare disease Reticulate acropigmentation of Kitamura Disease definition A rare, genetic, hyperpigmentation of the skin disease characterized by childhood to adulthood-onset of reticulate, slightly depressed, [orpha.net]

  Her brother also was found to have similar skin disease. The lesions were strictly confined to the hands and feet only suggesting the diagnosis of reticulate acropigmentation of kitamura. [ingentaconnect.com]

• Suggestibility

  There has been increasing reports of DDD–RAK overlap in the literature and some authors even suggest the term “Dowling–Degos syndrome” encompassing DDD, RAK, Haber syndrome, and acropigmentation of Dohi. [3], [4], [5], [11] However, there are suggestions [idoj.in]

  All the patients were female and the pattern of inheritance suggested an autosomal dominant mode. [ncbi.nlm.nih.gov]

  Interestingly, as this review was published a further series also suggests stability in FFA with prolonged dutasteride therapy. [typeset.io]

  The lesions were strictly confined to the hands and feet only suggesting the diagnosis of reticulate acropigmentation of kitamura. Its differentiating features from acropigmentation of Dohi and acromelanosis are described. [ingentaconnect.com]

• Facial Scar

  He also had multiple nonacne facial scars predominantly on the nose and malar areas. The patient had overlapping features of RAPK and DDD. In addition, he also had hypopigmented macules and acneiform facial scars. [ncbi.nlm.nih.gov]

• Lymphocytic Infiltrate

  Biopsy taken from dorsal aspect of forearm revealed histopathology almost similar to that of biopsy taken from nape of neck except mild perivascular lymphocytic infiltrates [Figure 2] b. These features are consistent with DDD. [idoj.in]

  In RAK the appearance resembles those seen in a solar lentigo with club-shaped elongations <strong>of</strong> the rete ridges but with intervening epidermal atrophy, melanin incontinence and perivascular lymphocytic infiltrate. [yumpu.com]

  A skin biopsy taken from the dorsa of the knee revealed hyperkeratosis, thinning of the epithelium, filiform elongation of the rete ridges and sparse lymphocyte infiltration at the dermis (Fig. 4). [escholarship.org]

  infiltrate, and an increased number of DOPA-positive basal melanocytes.4 There is some debate over whether RAPK, Dowling-Degos disease, Haber syndrome, and reticulate acropigmentation of Dohi are variants of the same disease, since their clinical and [actasdermo.org]

  infiltrate at the dermoepidermal junction, with occasional dyskeratosis and barely identifiable acantholysis Dysmaturative: disordered keratinocyte maturation with nuclear pleomorphism and slight hyperchromasia ( Am J Dermatopathol 2018;40:642 ) Epidermolytic [pathologyoutlines.com]

Treatment Options 1. Observation and reassurance 2. [dermatologyadvisor.com]

Unfortunately, there are no effective treatment options for these conditions. [ncbi.nlm.nih.gov]

primarily as a treatment for their coexisting androgenetic alopecia. [typeset.io]

Treatment  No specific treatment exists  Palmoplantar keratoderma: Topical keratolytics 45. [slideshare.net]

The treatment is symptomatic and involves skin therapy using topical applications and laser therapy, if necessary The prognosis is typically good for most individuals, since Dowling-Degos Disease is not a life-threatening condition. [dovemed.com]

Treatment and prognosis The disorder has a a poor prognosis and death is the rule, often in the third decade. [ijdvl.com]

1998 45 Co-existence of leukoderma with features of Dowling-Degos disease: reticulate acropigmentation of Kitamura spectrum in five unrelated patients. ( 9529553 ) Lestringant G.G....Galadari I.H. 1997 46 New protein and PCR markers RAK for diagnosis, prognosis [malacards.org]

A Chinese newborn with reticulate pigmented anomaly of the flexures was recently described. [21] Prognosis Dowling-Degos disease (reticulate pigmented anomaly) is slowly progressive but not life threatening. [emedicine.medscape.com]

Grover disease (GD) is a sporadic papular condition of uncertain etiology that presents most often in sun-damaged skin. Intractable pruritus is common. [accessmedicine.mhmedical.com]

Prurigo pigmentosa  Rare inflammatory disease of skin of uncertain etiology commonly reported from Japan  Recurrent eruptions of itchy urticarial macules, papules, vesicopapules, and plaques with a reticular arrangement  Quickly resolve leaving a net-like [slideshare.net]

Reviews addressing the etiology of vitiligo suggest that vitiligo is probably a heterogeneous disease encompassing multiple etiologies.47,48 An immune-mediated pathogenesis is the most popular theory. [what-when-how.com]

(Etiology) Dowling-Degos Disease is most commonly inherited from a single parent (in an autosomal dominant manner); however, the condition may also develop sporadically. [dovemed.com]

Although, the precise etiology of this disorder is not yet known, the dyschromatosis universalis hereditaria in an autosomal dominant disease but some cases are autosomal rescessive and the locus has been mapped to chromosome 6q24.2eq25.2 [ 3 ] and 12q21eq23 [clinical-pediatrics-dermatology.imedpub.com]

Each condition is organised into epidemiology, clinical evaluations, treatment and management. This new edition provides the most up-to-date information on each disorder. [books.google.com]

This chapter covers the epidemiology, pathophysiology, clinical presentation, histopathology, differential diagnosis, and treatment of both common and rare genetic hyperpigmentation disorders. [link.springer.com]

You might also be interested in Dermatology Occurrence/Epidemiology This section has been translated automatically. [altmeyers.org]

Epidemiology  Rare group of disorders  Incidence and prevalence: Not known  Most cases: Japan  Paucity of literature might be due to underreporting of cases  Mostly autosomal dominant with variable degrees of penetrance 6. [slideshare.net]

Epidemiology Frequency Dowling-Degos disease (reticulate pigmented anomaly) is a rare condition worldwide. Sex Dowling-Degos disease (reticulate pigmented anomaly) affects both sexes. [emedicine.medscape.com]

This chapter covers the epidemiology, pathophysiology, clinical presentation, histopathology, differential diagnosis, and treatment of both common and rare genetic hyperpigmentation disorders. [link.springer.com]

Etiology See Pathophysiology. Dowling-Degos disease (reticulate pigmented anomaly) may coexist with hidradenitis suppurativa. [emedicine.medscape.com]

It can be considered an acantholytic variant of Dowling-Degos disease. [ 8 ] The pathophysiology of Dowling-Degos disease is related to a genetic defect. [derm101.com]

The pigmentary system: physiology and pathophysiology. 2 nd ed. United States: Blackwell Science; 2006. p. 780-808. 8. Chang MW. Disorders of Hyperpigmentation. In: Rapini Ronald P, Bolognia Jean L, Jorizzo, Joseph L, editors. Dermatology. 2 nd ed. [ijdvl.com]

How can Dowling-Degos Disease be Prevented? Currently, there are no specific methods or guidelines to prevent Dowling-Degos Disease, since it is a genetic condition. [dovemed.com]

The patient's clinical photograph prevented us from making an embarrassing and uninformed diagnosis. Thanks to the referring dermatologist for providing such a beautiful clinical photograph. [dermpathmd.com]

Treatment  Exposure to heat should be limited, and sufficient hydration is recommended  Sports-related exercise should be restricted  Tooth care is required to prevent early dental caries 52. [slideshare.net]

Author Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public [emedicine.medscape.com]