|Year : 2017 | Volume
| Issue : 2 | Page : 109-111
Unilateral reticulate pigmentation of Kitamura
Gousia Sheikh, Yasmeen J Bhat, Muzaffar A Mir, Iffat Hassan
Postgraduate Department of Dermatology, STD & Leprosy, Government Medical College, University of Kashmir, Srinagar, Jammu and Kashmir, India
|Date of Web Publication||1-Dec-2017|
Postgraduate Department of Dermatology, STD & Leprosy, Government Medical College, University of Kashmir, Srinagar - 190 010, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
Reticulate acropigmentation of Kitamura (RAPK) is a rare genodermatosis characterized by pigmented, angulated, atrophic freckle-like lesions arranged in a reticulate pattern on the dorsal surface of hands and feet with symmetric involvement. We hereby report a case of unilateral RAPK in a 29-year-old female. The patient had cutaneous lesions and histopathology characteristic of RAPK, involving only the left side of her body with clear midline demarcation. This case is being reported in view of its rarity and being the first of its kind.
Keywords: Acropigmentation, genodermatosis, reticulate pigmentation, unilateral
|How to cite this article:|
Sheikh G, Bhat YJ, Mir MA, Hassan I. Unilateral reticulate pigmentation of Kitamura. Pigment Int 2017;4:109-11
| Introduction|| |
Reticulate pigmentary disorders are rare genetic abnormalities generally inherited in an autosomal dominant manner. These disorders include reticulate acropigmentation of Kitamura (RAPK), Dowling–Degos disease (DDD), reticulate acropigmentation of Dohi (RAPD), Haber’s syndrome, and Galli–Galli disease.
RAPK is clinically characterized by reticulate, slightly depressed pigmented macules on the acral parts of body (mainly the dorsa of the hands and feet). The lesions start in the first and second decades of life and gradually extend onto the extremities and rarely on the face and eyelids. The lesions usually darken slowly over time. Pits and breaks in the dermatoglyphics are found on the palms, soles, and dorsal phalangeal surfaces.
| Case report|| |
A 29-year-old female presented with asymptomatic, dark colored skin lesions on the left side of her body, including the face, neck, trunk, left upper and lower limb, and dorsum of left hand and foot since 8 years. There was no history of consanguinity. The lesions had first appeared on the dorsum of left hand and, over a period of 2 to 3 years, progressed to involve most of the left side of her body. There was no history of photosensitivity or prolonged intake of any drugs.
Dermatological examination revealed hyperpigmented, atrophic, angulated macules in a reticulate pattern involving only the left side of her body with a clear midline demarcation [[Figure 1],[Figure 2],[Figure 3]]. There was a relative sparing of flexures. Multiple pits with breaks in dermatoglyphics were present over the left palm only [Figure 4]. Mucous membranes and scalp were not involved. Hair was black in color, and nails and teeth were normal. Skin biopsy of a hyperpigmented macule over the trunk revealed elongation of rete ridges with increased number of melanocytes in basal layer of epidermis. Occasional melanophages were seen in dermis [Figure 5]. A diagnosis of unilateral reticulate pigmentation of Kitamura was made.
|Figure 3: Patchy reticulate angulated pigmented macules on left foot only|
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|Figure 4: Multiple pits on left palm with breaks in dermatoglyphics with sparing of right palm|
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|Figure 5: Increased number of melanocytes in basal layer with occasional melanophages in dermis (H&E 400X)|
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| Discussion|| |
RAPK was first described by Kitamura and Akamatsu in Japan. So far, near about 100 cases have been reported, mostly in dark skinned individuals of Asian ethnic origin. Clinically, RAPK is characterized by pigmented, angulated, slightly atrophic, and hyperpigmented macules over the distal extremities. On histopathological examination, epidermis shows atrophy with club-like elongation of the rete ridges and an excess of melanin in the basal layer.
The differential diagnosis of RAPK includes dyskeratosis congenita, dyschromatosis universalis hereditaria, Franceschetti–Jadassohn’s syndrome, dermatopathia pigmentosa reticularis, RAPD, and DDD [Table 1]. The differentiation between RAPD and RAPK is based on clinical and histological findings. In RAPK, there are usually no hypopigmented macules with histology showing epidermal atrophy and increase in the number of basal melanocytes. In DDD, reticular hyperpigmentation on flexor areas is seen and on histology pigmented filiform epidermal projections, involving the follicular infundibulum as well as the epidermis are seen. Comedo-like lesions and pitted perioral acneiform scars may also be present.
|Table 1: Differential diagnosis of reticulate acropigmentation of Kitamura|
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Many authors have proposed that DDD and RAPK may be an extended spectrum of a single entity, as numerous cases with overlapping features have been reported., However, molecular studies show mutations of KRT5 and POGLUT1, specifically in patients of DDD, whereas patients of RAPK are shown to have mutations in ADAM10. Still a group of patients with overlapping features of DDD and RAPK had mutations in POFUT1 gene.
Our patient had skin lesions which are characteristically seen in RAPK and the cutaneous biopsy also favored the diagnosis of RAPK. However, the involvement was both acral as well as nonacral. Specifically, our patient had a unilateral distribution of the lesions which is unheard of in RAPK. So our patient was a case with unilateral nonacral reticulate pigmentation of Kitamura. No significant treatment is available. Most treatments attempted for this disorder have been unsuccessful. However, treatment with 20% azelaic acid has shown significant improvement in some cases.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]