Letter to the Editor - Cutis

Letter to the EditorLetter to the EditorCoexistent Dowling-Degos Disease and ReticulateAcropigmentation of Kitamura With ProgressiveSeborrheic KeratosisTo the Editor:Reticular pigment anomaly of flexures, also calledDowling-Degos disease (DDD), is an autosomaldominantgenodermatosis characterized by reticulatepigmentation of the flexures. We report a case ofDDD concomitant with unusual clinical features. Inaddition to the typical flexural pigmented reticulatemacules, progressive seborrheic keratosis–like lesionson the affected flexural areas and reticulate acropigmentationof Kitamura (RAK) also were present.A 55-year-old woman presented with symptomaticreticulate hyperpigmentation on the neck,axillae, inframammary region, inguinal areas,of seborrheic keratosis–like lesions gradually developed,predominantly in the flexural pigmentedareas (Figure 1B). She also presented with pittedperioral scars. Her family history was positive for genodermatosisand some family members had similarpresentations. Histopathologically, thin branchingand elongation of rete ridges with basal hyperpigmentationwere observed in the right inguinal area.Neither perivascular lymphohistiocytic infiltrationnor acantholysis were found (Figure 2). A biopsyspecimen from one of the seborrheic papules showedtypical histopathologic features of the adenoid type(or reticulated type) of seborrheic keratosis includingepidermal thickening consisting of basaloidcells and multiple keratin-filled cysts (Figure 3).CUTISBlood, urine, and stool routine examinations; liverextremities, and the dorsa of the hands and feet and renal function tests; and blood pressure, were all(Figure 1). Head hair and nails were normal, but within reference range. Chest radiograph, abdomenaxillaryDohairs appeared scarce.NotThe eruptions began ultrasonographyCopystudies, and breast examinationsto develop in childhood and became more extensive were unremarkable. Tumor makers including carcinoembryonicantigen, cancer antigen (CA)–19-9,throughout adulthood. She did not report pruriticor painful sensations on the affected flexural areas, CA-242, a-fetoprotein, prostate-specific antigen,but she experienced pigmentation worsening after human growth hormone, neuron-specific enolaser,sun exposure. In the last 15 years, a large number b-human chorionic gonadotropin, free prostatespecificantigen, CA-125, and CA-15-3 werefound far below the upper limit of the referencerange. Nevertheless, the ferritin blood level wasABFigure 1. Clinical features of the reticulate pigmented eruption, including symptomatic reticulate hyperpigmentationon the axilla (A) and inguinal area (B). Seborrheic keratosis–like lesions gradually developed (inset)(B).WWW.CUTIS.COM VOLUME 87, FEBRUARY 2011 73Copyright Cutis 2011. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher.

Letter to the EditorCUTISDo Not CopyFigure 2. Light microscopy revealed typical thin branchingof epidermal downgrowths from one of the lesions ofreticulate hyperpigmentation (H&E, original magnification3100 [inset magnification 3400]).Figure 3. Light microscopy revealed many keratin-filledcysts from a seborrheic papule (H&E, original magnification3100 [inset magnification 3400]).250 ng/mL (reference range, 15–200 ng/mL). Theoverlap of DDD and RAK concomitant with progressiveseborrheic keratosis was diagnosed on thebasis of the clinical and histopathologic features.Although DDD and RAK are benign conditions,they usually cause aesthetically annoyingconsequences. 1 They are subsumed under theheading of reticulate hyperpigmentation and areinherited as a rare autosomal-dominant trait withvariable penetrance. 2 The association of these2 conditions has suggested the existence of aclinical spectrum between the DDD and RAKpoles 3 that could represent different features of thesame disease. Findings from our case strengthenthis hypothesis.Some disorders such as multiple epidermalcysts, hidradenitis suppurativa, and squamous cellcarcinomas have been reported in association withDDD-pigmented areas. 4 The coexistence betweenDDD and seborrheic keratosis does not seem tobe coincidental in this patient because severalmembers of her family were affected by both conditions.Seborrheic keratosis is one of the morecommon benign epidermal neoplasms; however,the association with malignancy can be useful asa cutaneous clue for diagnosis of occult internaltumors. 5 Therefore, one must emphasize the roleof correctly identifying its variants. The distinctclinical presentations in this patient concomitantwith progressive seborrheic keratosis may representan unusual variant of DDD associated with RAKand scarce axillary hairs. These clinical featuresmay reflect simultaneous underlying defects of follicularproliferation. Furthermore, the comparison74 CUTIS ® WWW.CUTIS.COMCopyright Cutis 2011. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher.

Letter to the Editorbetween the classic and variant forms may contributeto DDD gene mapping in special keratin genes. 6Characterization of the DDD gene will provideimportant clues to better understand the molecularmechanisms of pigmentation and would increasethe hope for effective interventional strategies inclinical practice.Chongqing. Dr. Santos is from the InternalMedicine Department of the Catholic UniversityMedical Course and Armed Forces Hospital,Brasília-DF, Brazil.The authors report no conflict of interest.REFERENCESAcknowledgment—This work was partly supported 1. Müller CS, Pföhler C, Tilgen W. Changing a concept—by the National Natural Science Foundation of controversy on the confusing spectrum of the reticulateChina (No. 81072502 and 30801013) and Innovation pigmented disorders of the skin [published online ahead ofProject of Outstanding Young Scholar of Third print June 17, 2008]. J Cutan Pathol. 2009;36:44-48.Military Medical University (No. 2007XG35).2. Wu YH, Lin YC. Generalized Dowling-Degos disease. JAm Acad Dermatol. 2007;57:327-334.Sincerely,3. Al Hawsawi K, Al Aboud K, Alfadley A, et al. ReticulateZhu Shen, MD, PhDacropigmentation of Kitamura-Dowling Degos diseaseLing Chen, MDoverlap: a case report. Int J Dermatol. 2002;41:518-520.Qingyi Ye, MD4. Weber LA, Kantor GR, Bergfeld WF. ReticulateFei Hao, MD, PhDpigmented anomaly of the flexures (Dowling-Degos disease):a case report associated with hidradenitis sup-Vitorino Modesto dos Santos, MD, PhDXichuan Yang, MD, PhDCUTISpurativa and squamous cell carcinoma. Cutis. 1990;45:Baiyu Zhong, MD446-450.5. Heaphy MR Jr, Millns JL, Schroeter AL. The sign ofDrs. Shen, Ye, Hao, Yang, and Zhong are from the Leser-Trélat in a case of adenocarcinoma of the lung. J AmDepartment of Dermatology, Southwest Hospital, Acad Dermatol. 2000;43(2, pt 2):386-390.Third Military Medical University, Chongqing, 6. Betz RC, Planko L, Eigelshoven S, et al. Loss-of-functionChina. Dr. Chen is from the Department of mutations in the keratin 5 gene lead to Dowling-DegosDermatology,DoInstitute of BattleNotSurgery, Daping diseaseCopy[published online ahead of print January 19, 2006].Hospital, Third Military Medical University, Am J Hum Genet. 2006;78:510-519.WWW.CUTIS.COM VOLUME 87, FEBRUARY 2011 75Copyright Cutis 2011. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher.